Rotor syndrome

Rotor syndrome
Bilirubin
ICD-10	E80.6
ICD-9	277.4
OMIM	237450
DiseasesDB	11671
MeSH	D006933

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Rotor syndrome is a rare, benign autosomal recessive disorder of unknown origin.

Presentation

It has many things in common with Dubin-Johnson syndrome except that in Rotor Syndrome, the liver cells are not pigmented. The main symptom is a non-itching jaundice. There is a rise in bilirubin in the patient's serum, mainly of the conjugated type.

	Rotor syndrome	DJS
appearance of liver	normal histology and appearance	liver has black pigmentation
gallbladder visualization	gallbladder can be visualized by oral cholecystogram	gallbladder cannot be visualized
total urine coproporphyrin content	high with <70% being isomer 1	normal with >80% being isomer 1 (normal urine contains more of isomer 3 than isomer 1)

Treatment

The liver is otherwise normal, and there is no need to treat this condition. It can be differentiated from Dubin Johnson syndrome by measuring the difference in urinary poryphrins. In Rotor syndrome there is an associated marked increase in urinary excretion of coproporyhrin I and III with < 80% being the I isomer.

Eponym

Rotor syndrome is named after the Filipino internist, Arturo Belleza Rotor (1907-1988).

See also

• Jaundice
• Bilirubin metabolism
• Dubin-Johnson syndrome
• Gilbert syndrome
• Crigler-Najjar syndrome

External links

• med/1065 at eMedicine - article on eMedicine on conjugated hyperbilirubinemia